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PYREXIA OF UNKNOWN ORIGINA DIAGNOSTIC DILEMMA
Dr. LAVANYA, DNB Post Graduate
Dr. JANANI SANKAR
KKCTH
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HISTORY• 4 years old/female child
• Developmentally normal
• Immunised for age
• No significant past illness
• H/O intermittent fever –1 month
• No other localising symptoms
• Had received multiple oral antibiotics
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EXAMINATION• Febrile, coated tongue.
• Abdomen – mild hepatomegaly.
• TC – 3100 (N 17,L 78), Hb – 9.4, Plat- 2.4L
• ESR - 40 mm/hr
• SGOT – 69 : SGPT – 19.
• Blood culture - sterile.
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TREATMENT• Partially treated enteric fever - 5 days of IV
Ceftriaxone
• She was afebrile for 24 hours
• Discharged on oral antibiotic (Cefixime) for a total duration of 2 weeks
• Plan was to repeat blood counts at review
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FOLLOW UP• Completed course of oral cefixime
• Persisting fever spikes – 8 weeks duration
• Repeat CBC - Persisting Leukopenia
(TC – 3100, Hb - 9.4, Plat – 2.4 L)
• Hence she was readmitted for further workup
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HISTORY - RELOOK
• H/O poor appetite – even before the illness
• No skin rashes/joint pain/joint swelling
• No H/O loss of weight
• No pallor / clinical bleeds
• No H/O contact with TB
• No H/O recent travel/contact with pets
• No H/O any drug intake
• No H/O surgery in past
• Vegetarian by diet – no H/O intake of any
• raw food
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EXAMINATION• Febrile , not sick looking• Pallor +• Painless Bilateral upper
eyelid swelling was seen• Glossitis +. No oral ulcers• No significant
lymphadenopathy• No skin rash/joint
swelling/bone tenderness• BCG scar + (No erythema)• Systems – mild
hepatomegaly
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DIFFERENTIAL DIAGNOSIS• Prolonged viral illness (EBV)
• Enteric fever
• Tuberculosis
• Collagen vascular disease
• Evolving malignancy
• Infection associated HLH
• Immunodeficiency – HIV
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WORK UP
• TC - 3,500 cells• DC - N 40/75, L 34/75, M 01/75• Hb – 7.6 g/dl • Platelet – 3.3 lakhs
• Peripheral smear – normocytic normochromic anemia, no evidence of hemolysis, no abnormal cells
• DCT – negative, Reticulocyte count - 1 %• Serum LDH – 3740 (elevated)
• ESR - 119 mm/hr
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• Renal function test - normal
• SGOT - 105 IU/L
• SGPT - 26 IU/L
• Serum albumin – 3.4 g/dl
• ECHO (to rule out Kawasaki disease / infective endocarditis) – Normal
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Work up for infective etiology
• EBV VCA IgM – negative
• WIDAL – negative
• Blood & Bone marrow culture – sterile
• Urine microscopy & culture – normal
• CSF analysis was normal
• Scrub typhus IgM – Non reactive
• HIV ELISA - Non reactive
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Work up for TB• ESR – elevated (119mm/hr)
• Mantoux – negative
• CXR – normal
• CT chest – normal. No mediastinal nodes
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Work up for malignancy• Peripheral smear – no abnormal cells
• Bone marrow smear – reactive marrow.
No atypical cells.
• USG abdomen – mild Hepatosplenomegaly
• Serum LDH – 3740
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Autoimmune workup• ANA – negative
• ds DNA - negative
Work up for infection associated HLH
• Ferritin – 898
• TGL – 329
• Fibrinogen – 375
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Management
• Temperature monitoring
• NSAID - Ibuprofen
• Empirical doxycycline (to cover any atypical organism)
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Ophthalmologist consult
‘BILATERAL DACRYOADENITIS’
?ORBITAL PSEUDOTUMOUR
underlying autoimmune disease or chronicinfection
Advised CT Orbit/oral steroid
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DACRYOADENITIS
• Inflammation of the lacrimal gland
• Acute or chronic
• Acute – Viral / Bacterial
• Chronic – Inflammatory disorders
• C/F: Painful swelling in the region of upper eyelid, fever, watering of eyes
• Inv: CT/MRI , Biopsy
• Treatment: Rest, warm compresses
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ORBITAL PSEUDOTUMOUR
• Idiopathic, nonmalignant orbital inflammation
• Etiology – Not known
Autoimmune etiology
• Present as a painful, unilateral orbital swelling
• Inv: MRI, biopsy
• Treatment: High dose steroids, immunosuppression, radiotherapy.
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THE FINAL CALL• Parents were given the option of further
workup – CT orbit and proceed or to start the child on steroid and look for the response.
•“NO” – For further evaluation
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• She was started on oral steroids.
• She was afebrilefor 2 days in the hospital.
• Discharged on steroids.
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FOLLOW UP
• She was afebrile.
• Eyelid swelling started resolving.
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Investigations at follow up
2 weeks after discharge
• TC – 8300 cells/cu.mm, Hb – 7.6g/dl
• Platelet count - 6.4Lakhs/cu.mm
• ESR – 108 mm/Hr
• ECHO (? Kawasaki disease) - normal
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Clinical diagnosis
? INCOMPLETE KAWASAKI DISEASE
• Started her on antiplatelet dose of aspirin.
• Advised to review in 2 weeks time.
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At review
• Clinically afebrile.
• No new signs or symptoms.
Repeat investigations:
• TC 6600, Hb 10, Platelets 3.3 L
• ESR -24 mm/Hr
• CRP was negative
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Possibilities:
• Can still be an incomplete KD, autoimmune disorder or evolving malignancy.
• She needs frequent follow up and
re-examinations to make a definitive diagnosis.
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ESR >100mm/hr
Tuberculosis
Kawasaki disease
Malignancy
Autoimmune disease
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Baseline
• CBC, ESR, CRP, Peripheral smear
• Mantoux
• Bone marrow smear
• Cultures – blood, urine, CSF, bone marrow
Serology
• Infectious mononucleosis, brucellosis, enteric fever, rickettsial, leptospirosis, lyme’s disease
• ANA, dsDNA, ANCA
Imaging
• Chest x-ray, USG Abdomen, CT chest, whole body MRI, PET
• Bronchoscopy, laparoscopy, mediastinoscopy, GI scopy
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TAKE HOME MESSAGES• Be rationale in investigating
a child with PUO.
• Don’t be in a hurry to label the child with a diagnosis.
• Counsel the family.
• YOUR PATIENCE IS ESSENTIAL.